Rheuma Flashcards

1
Q

most common rheumatic disease in children

A

Juvenile Idiopathic Arthritis (JIA)

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2
Q

criteria for JRA

A

age onset <16
arthritis (swelling or effusion or presence of 2 or more of the ff signs: limitation of range of motion, tenderness or pain on motion, increased heat) in >/= 1 joints
duration of >6weeks
polyarthritis >/=5 inflamed joints or
oligoarthritis =4 joints
exclusion of other forms of juvenile arthritis

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3
Q

systemic onset JIA

A
arthritis >/=1 joint with or preceded by fever of atleast 2 weeks documented to be quotidian for atleast 3 days
and accompanied by >/=1 of the ff:
*evanescent (nonfixed) erythematous rash
*generalized LN enlargement
*Hepatomegaly or splenomegaly or both
*serositis
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4
Q

Oligoarticular JIA

A

arthritis affecting 1-4 joints during 1st 6 months of disease

  • persistent- <4joints
  • extended- >5 joints
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5
Q

Psoriatic arthritis

A

Arthritis and Psoriasis or arthritis at least 2 of the ff:

  • dactylitis
  • nail pitting and oncholysis
  • psoriasis in a 1st degree relative
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6
Q

what is the risk for being ANA positive in patients with JIA?

A

increased risk for anterior uveitis

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7
Q

treatment for JIA

A

NSAIDs
Methotrexate
TNF-a antagonist

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8
Q

symptoms characteristic of inflammatory back pain

A

pain at night with morning stiffness
no improvement with rest
improvement with exercise
insidious onset

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9
Q

strongest risk factor for SLE

A

female

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10
Q

histopathologic feature most suggestive of SLE

A

discoid rash

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11
Q

hallmark of SLE

A

generation of autoantibodies directed against self-atigens

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12
Q

most feared complication of neonatal lupus

A

congenital heart block

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13
Q

heliotrope rash

A

blue-violet discoloration of eyelids

seen in Juvenile dermatomyositis

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14
Q

What are Gottron papules?

A

bright pink or pale, shiny thickened or atrophic plaques over proximal interphalangeal joints and DIP
Juvenile dermatomyositis

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15
Q

affects the proximal muscles with symmetric muscle weakness

A

Juvenile dermatomyositis

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16
Q

how do we diagnose Juvenile dermatomyositis?

A
characteristic rash and at least 3 of the ff:
muscle weakness
muscle enzyme elevation
EMG changes(myopathy, denervation)
muscle biopsy (necrosis, inflammation)
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17
Q

what is en coupe de sabre

A

morphea confined to frontal scalp, forehead and midface in a linear band may have seizure, hemifacial atrophy, ipsilateral uveitis, learning and behavioral changes (Scleroderma)

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18
Q

most common visceral manifestation of Scleroderma

A

Pulmonary disease

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19
Q

also known as mucocutaneous lymph node syndrome or infantile polyarteritis nodosa

A

Kawasaki disease

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20
Q

Kawasaki disease affects what arteries?

A

medium-sized arteries

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21
Q

most important manifestation of Kawasaki disease

A

cardiac involvement

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22
Q

wHat are the 3clinical phases of Kawasaki Disease

A

Acute febrile phase which lasts 1-2 weeks
Subacute phase: desquamation, thrombocytosis, developmentof coronary aneurysms lasts about 2 weeks
Convalescent phase:all clinical signs have disappeared and continues until ESR returns to normal

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23
Q

In Kawasaki disease, when do we expect the platelet count to increase?

A

second to third week of illness

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24
Q

In Kawasaki Disease, when echocardiography be done?

A

at diagnosis and at 2-3 weeks of illness

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25
Treatment for Kawasaki Disease
IVIg 2g/kg High dose Aspirin (80-100mkday q6) within 10 days onset decrease dose of Aspirin to 3-5mg/kg/day as single dose after being afebrile for 48 hours and continued until 6-8 weeks after illness onset
26
In patients who had Kawasaki disease and was given IVIG, when can we give live vaccines?
After 11 months
27
most common vasculitis of childhood
HSP
28
hallmark of HSP
rash: palpable purpura starting as pink macules or wheals developing into petechiae, raised purpura or larger ecchymoses
29
Classification criteria for HSP
ACR criteria: at least 2 of the ff: * palpable purpura * age onset <20 * bowel angina * biopsy demonstrating intramural granulocytes in small arterioles and/or venules European League against Rheumatism * palpable purpura * diffuse abdominal pain * arthritis or athralgia * biopsy of affected tissue demonstrating predominant IgA deposition
30
treatment for HSP
steroids
31
"pulseless disease"
Takayasu arteritis *subclavian, renal and carotid arteries mostly involved
32
criteria for Pediatric onset-takayasu arteritis
angiographic abnormalities of the aortaor its main branches and at least one of the ff: * decreased peripheral artery pulse and/or claudication * BP difference between arms or legs >10mmHG * bruits over aorta or its major branches * hypertension
33
mainstay of therapy for Takayasu
Glucocorticoids
34
necrotizing vasculitis affecting the small and medium sized arteries
Polyarteritis nodosa *conventional arteriography is the gold standard
35
found to be associated with neonatal lupus
Anti-Ro/SSA Anti-La/SSB Anti-RNP
36
cut-off value for ANA to diagnose SLE
>/= 1:80
37
What to do if mother is positive for anti-Ro and anti-la?
``` 2% offspring will develop neonatal lupus 2nd pregnancy 15-20% *regular fetal echo at 16 weeks of gestation until delivery *If (+) fetal incomplete block/hydrophic changes: fluorinated steroids IVIg plasmapharesis hydroxychloroquine Terbutaline + steroids given to mother ```
38
prognosis of neonatal LE
skin, liver and hematologic complications generally resolve with minimal sequalae All children with CAVB require pacemaker insertion by age of 15 heart block is permanent
39
Criteria for diagnosis of Behcet's Disease
recurrent oral ulcers plus 2 of the ff: * recurrent genital ulcers * eye lesions * skin lesions * pathergy - papular or pustular response after injection of NSS
40
treatment for Behcet's Disease
Azathioprine: eye disease Steroids, sucralfate: oral and genital ulcers Colchicine: erythema nodosum/arthritis (males), genital ulcers (females) Steroids, azathioprine, cyclophosphamide, interferon alpha,TNF blockers: CNS disease
41
Diagnosis of Immunoglobulin vasculitis (IgA)
Petechiae or purpura with lower limb predominance Plus >/= 1/4 of the ff: Diffuse abdominal colicky pain with acute onset (including intussusception and GI bleeding) Skin biopsy showing leukocytoclastic vasculitis with IgA deposits Arthritis or athralgia of acute onset Renal involvement (proteinuria >0.3g in 24 hours, hematuria or RBC casts, impaired renal function
42
common presenting complaint in JDM
Fatigue
43
Most common inflammatory myositis in children distinguished by proximal weakness and characteristic rash
JDM
44
Diagnostic criteria of JDM
``` Classic rash plus 3 of the ff: Weakness Muscle enzyme elevation Electromyographic changes Muscle biopsy ```
45
Blue violet discoloration of eyelids
Heliotrope rash seen in | JDM
46
most common visceral manifestation of systemic sclerosis
Pulmonary disease
47
most frequent initial symptom of in pediatric systemic sclerosis
Raynaud phenomenon
48
vasospastic disorder resulting in cool, painless, bluish discoloration in the hands and sometimes feet despite normal tissue perfusion
Acrocyanosis
49
most common cause of death in Scleroderma
heart failure
50
confined to frontal scalp, forehead and midface in linear band
en coup de sabre
51
most common rheumatic disease in children
JIA
52
most common subtype of JIA
Oligoarthritis
53
most common affected joints in Enthesitis-Related Arthritis
Knees, ankles and hips
54
most common presenting complaints of children with SLE
fever, fatigue, hematologic abnormalities, arthralgia and arthritis
55
most common cause of death in patients with SLE
infection, complications of GN and neuropsychiatric disease
56
most common subtype of Juvenile localized scleroderma
Linear scleroderma
57
most common manifestations of Sjogren syndrome in children
recurrent parotid gland enlargement and parotitis
58
most common manifestations of Sjogren syndrome in adults
Sicca syndrome (dry mouth, painful mucosa, sensitivity to spicy foods, halitosis, widespread dental caries
59
most common vessels affected in Kawasaki disease
coronary arteries
60
aortic branches affected in Takayasu arteritis
subclavian renal carotid arteries
61
most common presenting problem of children referred to pediatric rheumatology clinics
musculoskeletal pain
62
most common cause of recurrent musculoskeletal pain in children
Benign nocturnal pains of chidlhood (growing pains)